Biomedical subjects
Publications and source records attributed to G Goerz.
Hairless female Ng/-mice were irradiated by UV-light for 16 h daily over a period of 24 weeks. Monooxygenase activities were measured in liver and skin, and an induction of the aryl-hydrocarbon hydroxylase was detected in liver by both fluorometric and radiochemical methods, whereas no induction of this enzyme could be demonstrated in the skin.
Reactions as intolerance to aspirin and food additives were diagnosed in 41 cases during November 1979 -- March 1982. Allergy to pyrazolone-drugs were observed in 20 cases during the same period. 24% of the patients with intolerance to aspirin had also an intolerance to tartrazine. There were no familial occurrence of aspirin intolerance. Four patients had mainly asthma, 37 patients urticaria. New aspects of the pathogenesis of aspirin intolerance -- modulation of arachidonate metabolism and complement activation -- are discussed.
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We examined the efficacy of cimetidine and chlorpheniramine alone and in combination in the treatment of psoriasis under the conditions of a randomised controlled double-blind study. Analysis of data from 52 patients revealed that none of the treatment regimes showed a demonstrable beneficial effect on the course of the psoriasis.
A female patient with porphyria cutanea tarda (PCT) clinically cured by a low-dose chloroquine treatment showed neither exacerbation of the disease nor an increased excretion of urinary porphyrins during pregnancy. The role of estrogens and gestagens as clinical manifestation factors of PCT is discussed. According to our observation the porphyrogenic effect of the estrogens seems to have been overestimated.
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There is a controversial discussion of the carcinogenic action of coal tar used as a therapeutic agent in dermatologic practice. The carcinogen benzo(a)pyrene is present in most coal tar preparations, and it is a potent inducer of the aryl hydrocarbon hydroxylase activity in liver and skin after topical application. The formation of the most reactive metabolite of benzo(a)pyrene is catalyzed by aryl hydrocarbon hydroxylase hydroxylase. Topically used coal tar alters the mutagenicity of the urine indicating a systemic effect. These experimental data recommend to be very cautious in using coal tar as a therapeutic agent although there are only a few case reports on tumors after treatment with coal tar.
Recent biochemical advances have contributed to clarification of certain skin diseases and metabolic disturbances with predominantly cutaneous symptoms. This is illustrated by the various forms of porphyria. Today we differentiate four hepatic forms: acute intermittent porphyria, variegate porphyria, hereditary coproporphyria and porphyria cutanea tarda, and two erythropoietic forms: congenital erythropoietic porphyria and erythropoietic protoporphyria, all of which are due to an inborn enzymatic deficiency of the heme biosynthesis. From the different forms of ichthyosis, the X-recessive ichthyosis has an underlying enzymatic deficiency of the steroid sulfatase, which seems of significance in the disturbance of keratinization. In epidermolysis bullosa dystrophica type Hallopeau-Siemens an increased collagenase activity was detected. Inhibition of this enzyme by phenytoin results in improvement of the blistering in this genodermatosis. The etiology and pathogenesis of psoriasis are unclear despite extensive efforts. The recently detected deficiency of the arylhydrocarbon-hydroxylase and its inducibility must be confirmed, additionally its significance in the pathogenesis of this disease is yet to be evaluated.
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Adult female Wistar rats were fed continuously over a period of 65 days with a diet containing 0.05% hexachlorobenzene (HCB), silymarin (100 mg/kg) or (+)-cyanidanol-3 (150 mg/kg) alone or together with HCB. On day 49 the porphyrins were increased in the cyanidanol-HCB-group in contrast to the silymarin-HCB- and the HCB-group which showed lower levels during the whole experiment. Compared with the other groups--aminolevulinic acid and porphobilinogen were increased in the cyanidanol/HCB-group. Silymarin and cyanidanol had no effect on the formation of experimental porphyria. On the other hand, silymarin is a stronger inducer of the mixed function monooxygenase system than cyanidanol. The type of the cytochrome P-450 induced by the substances could not be identified with the help of different type-specific inhibitors.
The pharmacology of the most important immunosuppressive agents in dermatology: glucocorticoids, azathioprine, cyclophosphamide, methotrexate and chlorambucil are reviewed. Our own results of treatment with these drugs (102 patients, diagnosis: lupus erythematosus, dermatomyositis, scleroderma, overlap-syndrome, pemphigus vulgaris, bullous pemphigoid, cryoglobulinaemic purpura, and pyoderma gangraenosum) are presented.
Scleroedema adultorum Buschke is characterized by progressive hardening of the skin. In contrast to scleroderma the hardening occurs in the skin of the trunk while extremities remain largely free. Internal organs are said not to be involved in scleroedema adultorum Buschke. The full picture of the persistent form of scleroedema adultorum Buschke was observed in two patients. One patient complained of increasing dysphagia with regurgitation and aspiration. Manometry and X-ray cinematographic investigation showed inappropriate relaxation of the upper oesophageal sphincter. In the other patient who had not previously had swallowed difficulties manometry showed achalasia of the upper oesophageal sphincter. The functional disturbances of the upper oesophagus indicate the possibility of an involvement of internal organs in scleroedema adultorum Buschke. However, proof of an aetiological connection between disturbances of oesophageal motility and skin disease requires systematic investigations in a larger group of patients.
The case of a 29 year old man is presented, who showed 'sunburn like' reactions following short exposures to light since early childhood. Demonstration of fluorocytes and analysis of porphyrins confirmed the diagnosis of erythropoietic protoporphyria (EPP). It is noteworthy that the patient failed to show clinical signs, characteristic for EPP at the nasal area or the extensor aspects of the hands, whereas indurated plaques with firm skin coloured papules were observed in the shoulder regions. Biopsies taken from these areas revealed PAS-positive material especially around the capillaries of the dermal papillae, a finding typically for EPP. Electronmicroscopic investigation revealed reduplication of the vascular basal lamina and perivascular deposition of a fine fibrillar material. Therefore the cutaneous changes, yet clinically atypical, seem to be correlated with EPP.
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A monoclonal gammopathy was observed in three patients with long-term and widespread scleredema (Buschke's disease). There was no evidence of multiple myeloma in any patient. Deposition of monoclonal immunoglobulins in the skin was not detected by direct immunofluorescence microscopy. In contrast to scleromyxedema (lichen myxedematosus), from which scleredema can be distinguished clinically and histologically, the monoclonal immunoglobulins in two cases were of IgG2-kappa and IgG3-kappa type. Only one of the three patients had IgG1-lambda paraproteinemia, which is frequently seen in scleromyxedema. Our findings suggest that diffuse scleredema may be characterized by paraproteinemia but that the possible role of monoclonal immunoglobulins in the pathogenesis of this disease has yet to be resolved.
A 5-year-old girl with the characteristic clinical picture of "spun glass hair" is described; additionally she suffers from atopic dermatitis. This case is of special interest since the child not only suffers also from endogenic eczema, but that another member of the family (child's aunt) also suffers from the same hair anomaly. The diagnosis is based upon the clinical symptom that the hair cannot be managed, and is proved by observation of triangular and kidney-shaped cross-sections taken from the child's hair. Electron scan microscopic examination of the hair revealed grooves on the surface of the hair which exhibit similarity to the hair-condition pili canaliculi. The following physical properties of these irregularly shaped hairs were measured: Fibre thickness, tensile strength, extent of fibre expansion shortly before breakage, and form elasticity. None of these properties were found to differ from those of normal hair.